Introduction: Total anomalous pulmonary venous connection (TAPVC) has a low prenatal diagnostic rate. Therefore, we investigated whether Doppler waveforms with a low pulsatility in the pulmonary veins can indicate fetal TAPVC. Methods: This retrospective study included 16 fetuses with TAPVC, including 10 with complex congenital heart disease and 104 healthy fetuses that underwent fetal echocardiography. Pulmonary venous S and D wave flow velocities and the valley (representing the lowest velocity between the S and D waves) were measured. Valley indices I and II were then calculated as (velocity of valley/greater of the S and D wave velocities) and (velocity of valley/lesser of the S and D wave velocities), respectively. Results: Supra/infracardiac TAPVC cases exhibited significantly greater valley indices than that of the healthy group. After adjusting for gestational age at fetal echocardiography, valley indices I (odds ratio [OR] 7.26, p < 0.01) and II (OR: 9.23, p < 0.01) were significant predictors of supra/infracardiac TAPVC. Furthermore, valley indices I and II exhibited a high area under the curve for detecting supra/infracardiac TAPVC, regardless of the presence of pulmonary venous obstruction. Conclusion: The valley index may be a useful tool for the detection of fetal TAPVC.

Total anomalous pulmonary venous connection (TAPVC) requires urgent intervention after birth. Furthermore, prenatal diagnosis is essential to improve the postnatal clinical course. However, fetuses with TAPVC show no identifying characteristics of the disease in echocardiographic images, producing a low prenatal diagnostic rate. Our research proposes a “valley index” to objectively quantify the low pulsatility of pulmonary venous waveforms in fetuses and serve as an indicator of fetal TAPVC. The study sample consisted of 16 fetuses with TAPVC, including 10 with complex congenital heart disease and 104 healthy fetuses. The valley index was calculated using velocities of the S wave, D wave, and valley between the waves. The valley indices were significantly higher in the groups with supracardiac and infracardiac TAPVC than in the healthy control group; indicating that it was a significant predictor of the two types of TAPVC. The optimal cutoff value of the valley index was calculated, showing high sensitivity and specificity for the detection of fetal TAPVC. Therefore, the valley index may be a useful tool for the prenatal detection of TAPVC.

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